Differential levels of Neurofilament Light protein in cerebrospinal fluid in patients with a wide range of neurodegenerative disorders.

Cerebrospinal fluid (CSF) biomarkers are useful in the diagnosis and the prediction of progression of several neurodegenerative diseases. Among them, CSF neurofilament light (NfL) protein has particular interest, as its levels reflect neuroaxonal degeneration, a common feature in various neurodegenerative diseases. In the present study, we analyzed NfL levels in the CSF of 535 participants of the SPIN (Sant Pau Initiative on Neurodegeneration) cohort including cognitively normal participants, patients with Alzheimer disease (AD), Down syndrome (DS), frontotemporal dementia (FTD), amyotrophic lateral sclerosis (ALS), dementia with Lewy bodies (DLB), progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS). We evaluated the differences in CSF NfL accross groups and its association with other CSF biomarkers and with cognitive scales. All neurogenerative diseases showed increased levels of CSF NfL, with the highest levels in patients with ALS, FTD, CBS and PSP. Furthermore, we found an association of CSF NfL levels with cognitive impairment in patients within the AD and FTD spectrum and with AD pathology in DLB and DS patients. These results have implications for the use of NfL as a marker in neurodegenerative diseases.

[(123)I-MIBG myocardial scintigraphy in the diagnosis of Lewy body dementia]. / Gammagrafía miocárdica con I-MIBG en el diagnóstico de la demencia con cuerpos de Lewy.

INTRODUCTION: Lewy body dementia (LBD) is the second most common cause of neurodegenerative dementia after Alzheimer's disease (AD). A cardiac post-ganglionic sympathetic denervation has been described in this condition which can be quantified by MIBG (metaiodobenzylguanidine) myocardial scintigraphy. The aim of our work was to retrospectively evaluate cardiac MIBG uptake (expressed as the heart-to-mediastinum ratio at 4h (HMR) in patients with suspected LBD, and to examine its relationship with clinical and para-clinical data. MATERIAL AND METHODS: A total of 77 patients with clinical suspicion of LBD evaluated at our centre between September 2005 and June 2008 to whom a MIBG myocardial scintigraphy has been performed were retrospectively reviewed. International Consensus Criteria of LBD were applied to divide the sample into probable LBD, possible LBD and non-LBD. HMR values and their relationships with clinical and neuropsychological data were analysed. A subgroup of patients had FP-CIT (fluoropropyl-carbomethoxy-3ß-4-iodophenyltropane) SPECT as a part of the evaluation. RESULTS: Mean HMR values were significantly lower in probable LBD group than in possible LBD and non-LBD groups. Low HMR values were associated only with reduced FP-CIT uptake in the striatum, but not with any clinical or neuropsychological item. CONCLUSIONS: Low MIBG myocardial scintigraphy uptake is a robust measure in LBD, and it is not largely affected by medical conditions, or by the stage of the disease. In LBD reduced MIBG myocardial uptake is associated with nigrostriatal degeneration.

Gammagrafía miocárdica con 123I-MIBG en el diagnóstico de la demencia con cuerpos de Lewy / 123I-MIBG myocardial scintigraphy in the diagnosis of Lewy body dementia

Introducción: La demencia con cuerpos de Lewy (DCLw) es la segunda causa más frecuente de demencia degenerativa tras la demencia tipo Alzheimer (DTA). En esta entidad se ha descrito una denervación simpática cardíaca posganglionar, que puede cuantificarse mediante la gammagrafía miocárdica con MIBG (metayodobencilguanidina). El objetivo de nuestro trabajo fue evaluar retrospectivamente la captación miocárdica de MIBG, expresada cuantitativamente como el índice corazón/mediastino a las 4h (ICM) en pacientes con sospecha clínica de DCLw, y examinar su relación con los datos clínicos y paraclínicos. Pacientes y métodos:Se revisaron retrospectivamente datos de 77 pacientes con sospecha clínica de DCLw evaluados entre septiembre de 2005 y junio de 2008 en nuestro hospital a los que se les había realizado una gammagrafía miocárdica con MIBG. Se aplicaron los criterios internacionales de consenso para dividir la muestra en DCLw probable, DCLw posible y sin DCLw. Se analizaron el ICM en cada grupo y su relación con variables clínicas y neuropsicológicas. A un subgrupo de pacientes se le había realizado además un SPECT con FP-CIT (fluoropropil-carbometoxi-3β-4-yodofeniltropano) como parte de la evaluación. Resultados:Los valores medios de ICM fueron significativamente menores en el grupo de DCLw probable que en los grupos de DCLw posible y sin DCLw. Valores disminuidos de ICM solamente se asociaron a una captación disminuida en el estriado en el FP-CIT SPECT, pero no a ninguna variable clínica ni neuropsicológica. Conclusiones:La captación miocárdica reducida de MIBG es una medida robusta en la DCLw, y no se ve afectada por condiciones médicas o el estadio de la enfermedad. Una disminución en la captación miocárdica de MIBG se asocia a la degeneración nigroestriada en la DCLw (AU)

Introduction: Lewy body dementia (LBD) is the second most common cause of neurodegenerative dementia after Alzheimer's disease (AD). A cardiac post-ganglionic sympathetic denervation has been described in this condition which can be quantified by MIBG (metaiodobenzylguanidine) myocardial scintigraphy. The aim of our work was to retrospectively evaluate cardiac MIBG uptake (expressed as the heart-to-mediastinum ratio at 4h (HMR) in patients with suspected LBD, and to examine its relationship with clinical and para-clinical data. Material and methods: A total of 77 patients with clinical suspicion of LBD evaluated at our centre between September 2005 and June 2008 to whom a MIBG myocardial scintigraphy has been performed were retrospectively reviewed. International Consensus Criteria of LBD were applied to divide the sample into probable LBD, possible LBD and non-LBD. HMR values and their relationships with clinical and neuropsychological data were analysed. A subgroup of patients had FP-CIT (fluoropropyl-carbomethoxy-3β-4-iodophenyltropane) SPECT as a part of the evaluation.Results: Mean HMR values were significantly lower in probable LBD group than in possible LBD and non-LBD groups. Low HMR values were associated only with reduced FP-CIT uptake in the striatum, but not with any clinical or neuropsychological item.Conclusions: Low MIBG myocardial scintigraphy uptake is a robust measure in LBD, and it is not largely affected by medical conditions, or by the stage of the disease. In LBD reduced MIBG myocardial uptake is associated with nigrostriatal degeneration (AU)